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Systemic Juvenile Idiopathic Arthritis

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Abstract

Systemic-onset juvenile idiopathic arthritis is a complex, polygenic autoinflammatory disease affecting young individuals. It is an innate immunity-driven condition characterized by chronic, multiorganic inflammatory involvement. Typical clinical features including young age at presentation, fever, neutrophilic skin lesions, synovitis, serositis, organomegaly, as well as increased risk of amyloidosis are reminiscent of (and may mimic) certain monogenic autoinflammatory disorders. Strikingly high acute phase reactants, thrombocytosis, hyperferritinemia, and elevated serum levels of inflammatory-related proteins such as IL-1, IL-18, S100A8/9, and S100A12 characterize active phases. The disease may follow a relapsing or a continuous course into adulthood, frequently leading to joint damage, disability, and poorer quality of life. Major morbidity and mortality arise from the hypercytokinemic, hyperinflammatory stages called macrophage activation syndrome, a form of reactive hemophagocytic lymphohistiocytosis, which may occur in 50% of patients but become clinically overt only in a smaller proportion. Multidisciplinary teams are best fit to treat this complex disease. Decades after nonbiologic drugs and TNF inhibitors had consistently failed, the advent of biologic agents blocking IL-1 and IL-6 has proven dramatically efficacious for medical management.

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  1. Service of Immunology & Rheumatology, Hospital de Pediatría Prof. Dr. Juan P. Garrahan, Buenos Aires, Argentina

    Ricardo A. G. Russo & María Martha Katsicas

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  1. Ricardo A. G. Russo
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    Petros Efthimiou

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Russo, R.A.G., Katsicas, M.M. (2019). Systemic Juvenile Idiopathic Arthritis. In: Efthimiou, P. (eds) Auto-Inflammatory Syndromes. Springer, Cham. https://doi.org/10.1007/978-3-319-96929-9_13

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